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Congenital Defects & Pediatrics IA > TGA- Topic 15 > Flashcards

TGA- Topic 15 Flashcards

1
Q

TGA/TGV

A

Discordant ventricular-arterial relationship

Malformation in which the two great arteries carrying blood away from the heart are transposed or reversed

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2
Q

The LV –>

A

PA

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3
Q

The RV –>

A

Aorta

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4
Q

What’s the main classification of CHD?

A

Acyanotic

Cyanotic

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5
Q

Two types of Acyanotic CHD

A

Increased pulmonary blood flow

Obstruction to blood flow from ventricles

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6
Q

Two types of Cyanotic CHD

A

Decreased blood flow

Mixed blood flow

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7
Q

Acyanotic: Increased Pulmonary Blood Flow

A

ASD
VSD
PDA
AV Canal

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8
Q

Acyanotic: Obstruction to blood flow from ventricles

A

Coarc of aorta
Aortic stenosis
Pulmonic stenosis

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9
Q

Cyanotic: Decreased pulmonary blood flow

A

TOF

Tricuspid Atresia

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10
Q

Cyanotic: Mixed Blood Flow

A

TGA
TAPVR
TA
HLHS

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11
Q

TGA is compatible with life unless what happens?

A

Some communication exists between the two separate circulatory systems.

Frequently patients with TGA have ASD’s or VSD’s

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12
Q

What are the two parallel circulations in TGA?

A

Body- RA - RV- Ao-Body

Lungs- LA- LV- PA- Lungs

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13
Q

What type of mixing in TGA?

A

Poor mixing

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14
Q

What are some symptoms of TGA?

A 
Hypoxia & Acidemia
Hyperventilation
Increased pulmonary flow
CHF
Myocardial depression
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15
Q

What is the most common cyanotic congenital heart lesion presenting in the neonate?

A

TGA

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16
Q

What percent of cases are TGA?

A

5%

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17
Q

TGA: Gender

A

More common in males, with a ratio of about 3:1

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18
Q

What are some maternal factors that are associated with an increased of TGA?

A

Rubella or other virus during pregnancy
Alcoholism
Maternal age over 40
Diabetes

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19
Q

TGA is a defect of the …..

A

Bulbus cordis

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20
Q

TGA: After outflow tract septation development beings then improper spiraling of _____________. This leads to…..

A

the aorticopulmonary septum
This leads to congenital disruption in pulmonary and systemic circulations (sounds a bit like the TOF defect development)

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21
Q

Truncus Arteriosus becomes…..

A

Aorta

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22
Q

Conus Cordis becomes… What is this created by?

A

Pulmonary Artery; created by a septum that forms in the outflow tract from these swellings

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23
Q

What day is outflow tract septation?

A

Day 29

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24
Q

As is with TAPVR, without intervention infants with TGA will die within how long?

A

Their first year of life

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25
Q

What are the two anatomical classifications of TGA?

A

D-Transposition (Right)

L-Transposition (Left)

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26
Q

In what percent of patients is the aorta anterior and to the right of hte pulmonary artery?

A

60%

Dextro-transposition of the great arteries (d-TGA)

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27
Q

What is called when the aorta is anterior and to the left of the pulmonary artery?

A

(levo-transposition of the great arteries (l-TGA)

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28
Q

What is the only distinguishing characteristic that defines TGA?

A

Discordant ventriculo-arterial connection

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29
Q

What are presentations common to TGA?

A
  1. TGA w/ intact ventricular septum
  2. TGA w/ VSD
  3. TGA w/ VSD and LVOT obstruction
  4. TGA w/ VSD and Pulmonary vascular obstructive disease
30
Q

TGA first described over 200 years ago tx was not available until:

A
  1. development of surgical atrial septectomy in the 1950’s

Balloon atrial septostomy in the 1960’s.

31
Q

Palliative therapies followed by:

A 
Physiological procedures (atrial switch operation)
Anatomic repair (arterial switch operation)
32
Q

What is the survival rate for infants with TGA?

A

Greater than 90%

33
Q

What is the initial treatment consist of?

A

Ductal patency with continuous IV prostaglandin E1 infusion (PGE1)

34
Q

What does continuous IV prostaglandin E1 infusion PGE1 result in?

A

Increased pulmonary blood flow
Increased left atrial pressure
Promotes L –> R shunting at the atrial level (decreased cyanosis)
Impt in patients with severe LVOT (PDA will not help when the defect has intact septum’s and separate circulations)

35
Q

Why might an arterial switch operation not be feasible in TGA w/ IVS and LVOT obstruction?

A

Pulmonary (LVOT) stenosis or atresia

36
Q

If the VSD is nonrestrictive and not too remote from the aorta, what repair could be possible?

A

Rastelli intracardiac repair

37
Q

Rastelli Procedure

A

conduit from the RV to the PA, delaying repair
*placing an aorto-pulmonary shunt during the newborn period may be necessary to establish adequate pulmonary blood flow while waiting (i.e. central shunt)

38
Q

What are the two types of atrial switch procedures?

A

Mustard

Senning

39
Q

What are the two types of arterial switches?

A

Jatene

Le COmpte

40
Q

Mustard procedure- atrial baffle

A

Restores circulation, but reverses the direction of the blood flow in the heart
Blood is pumped to the lungs via the LV and disseminated throughout the body via the RV
But the right ventricle is not the optimal shape to support the high pressure work performed in an ormal heart by the LV

41
Q

What type of baffle does the mustard procedure use?

A

Pericardial tissue baffle

42
Q

Senning Procedure

A

A baffle is created within the atria that redirects the deoxygenated caval blood to the mitral valve and the oxygenated pulmonary venous blood to the tricuspid valve

The anatomic LV continues to act as the pulmonary pump and the anatomic RV acts as the systemic pump

43
Q

What is the dfiference beween the Mustard procedure and Senning procedure?

A

Identical except that the baffle is constructed from atrial tissue in the Senning and from pericardium in the Mustard

44
Q

What is the standard therapy for transposition?

A

The neonatal arterial switch procedure; by the 1980’s it was adopted

45
Q

The Mustard procedure was replaced in the late 1970’s by what procedure?

A

Jatene procedure (arterial switch)

46
Q

Jatene Procedure

A

Native arteries are switched back to normal flow, so that the RV would be connected to the pulmonary artery and the LV would be connected to the aorta

47
Q

Why was the Jatene procedure not possible prior to 1975?

A

because of the difficulty with re-implanting coronary arteries which perfuse myocardium

48
Q

CPB Considerations TGA: Cannulation

A

Arterial: Aortic
Venous: Single Atrial (bicaval if 4+ kg)

49
Q

CPB Considerations TGA: Hypothermia

A

DHCA/Low flow w/ HCA

50
Q

CPB Considerations TGA: Cardioplegia

A

Antegrade, Retrograe, Ostial (multiple dosing)

51
Q

What type of weight do TGA kids have?

A

These children are larger weight; around 3 kg

52
Q

Is complete correction or palliation more common to tx TGA?

A

Complete correction

53
Q

is the post-procedure for TGA open or close?

A

Open chest-post procedure (silastic patch)

54
Q

What is the length of the procedure to correct TGA?

A

Longer procedure, technically difficult

55
Q

Truncus Arteriosus AKA

A

persistent truncus arteriosus

56
Q

Truncus Arteriosus

A

a rare type of congenital heart disease comes out of hte right and left ventricles, instead of the normal two (pulmonary artery and aorta)

57
Q

In TA is untreated what two problems occur?

A
  1. Too much pulmonary flow
  2. The blood vessels to the lungs become permanently damaged. Pulmonary hyptertension develops; the major problem in Truncus is that the lungs are flooded w/ blood and the heart muscle is overloaded
58
Q

TA Symptoms

A 
Bluish skin (cyanosis)
Delayed growth or growth failure
Fatigue
Lethargy
Poor feeding
Rapid breathing (tachypnea)
Shortness of breath (dyspnea)
Widening of the finger tips (clubbing)
59
Q

What causes TA?

A

Failed septation of the embryonic truncus arteriosus. Aortiopulmonary and interventricular defects are believed to represent an abnormality of conotruncal septation.

Because of the common trunk originates from both the LV and RV, and PAs arise directly from the common trunk, a PDA is not required to support the fetal circulation

60
Q

What divides the truncus arterosus and bulbus cordis?

A

Aortico-pulmonary septum

61
Q

Truncus arteriosus gives rise to what?

A

The ascending aorta and the pulmonary trunk

62
Q

What does the bulbus cordis give rise to?

A

The smooth parts (outflow tract) of the LV and RV

63
Q

TA: Type I

A

Truncus –> One pulmonary artery –> Two lateral pulmonary arteries

Origin of a single pulmonary trunk from the left lateral aspect of hte common trunk, with branching of hte left and right pulmonary arteries form the pulmonary trunk

64
Q

TA: Type II

A

Truncus –> two poster/posteriolateral pulmonary arteries

separate but proximate origins of hte left and right pulmonary arterial branches from the posterolateral aspect of hte common arterial trunk

65
Q

TA: Type III

A

Truncus –> two lateral pulmonary arteries

Branch pulmonary arteries oringinate independently from the common arterial trunk or aortic arch (most often from the left and right lateral aspects of hte trunk)

This occasionally occurs with origin of one pulmonary artery from the underside of hte oartic arch, usually from a ductus arteriosus

66
Q

Palliative Surgery for TA

A

PA Banding; left anterior thoracotomy approach through the second or third intercostal space gives excellend exposure for isolated PAB

Extracardiac procedure -no CPB

67
Q

What is the treatment of choice for TA?

A

Complete repair and usually a modification of a Rastelli procedure. A Rastelli procedure connects the RV-PA. This tube is usually a homograft (made a human vadaver tissue)

During the Rastelli procedure, the VSD is closed with a Core-Tex patch so that hte oarta arises solely from the left ventricle

68
Q

CPB TA: Incision

A

Median Sternotomy

69
Q

CPB TA: Cannulation

A

Arterial - Aortic

Venous-Single Atrial cannula

70
Q

CPB TA: XC

A

moderate lenght

71
Q

CPB TA: hypothermia

A

mild to moderate period DHCA

72
Q

CPB TA CPG

A

antegrade possibly retorgrade.

Congenital Defects & Pediatrics IA (17 decks)

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